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GDP-D-glucose phosphorylase 1 is a specific and highly efficient enzyme responsible for the breakdown of GDP-D-glucose to GDP and D-glucose 1-phosphate[1][2][5][4][6]. It acts primarily in the cytoplasm[4]. Its main physiological role is to regulate cellular GDP-D-glucose levels, thereby preventing the erroneous incorporation of glucose—rather than mannose—into glycosylated molecules during glycan assembly[1][2][6]. Through this activity, it contributes to proper glycosylation and metabolic homeostasis in both mammals and invertebrates. Mutations in this gene (GDPGP1, also previously known as C15orf58) can alter normal glycosylation patterns, with emerging evidence for roles in rare metabolic or developmental disorders and potentially in neuroprotection[7][4][6]. No specific drugs targeting GDPGP1 are currently known.
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